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  • 1
    ISSN: 1749-6632
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Natural Sciences in General
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Alzheimer's disease ; Granulovacuolar degeneration ; Hippocampus ; Paired helical filament ; Tau protein
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An immunocytochemical study of Alzheimer's disease hippocampus with a panel of anti-tau antibodies revealed two antibodies that stained granulovacuolar bodies (GVB) in pyramidal neurons of Ammon's horn. These two affinity-purified anti-tau antibodies were raised in rabbits against synthetic peptides homologous to sequences (amino acids 44–55 and 75–87) in the 58 amino acid insert in the amino terminus of the longest form of human tau. This region is homologous to exons 2 and exon 3 of bovine tau. The exon 2 peptide contains a serine (amino acid residue 46), which has been shown to be a phosphorylated site in paired helical filaments. Antibodies to a nonphosphorylated exon 2 peptide failed to immunostain GVB, but those to the phosphopeptide consistently stained GVB. Staining, however, was most consistent with the antibody to the exon 3 sequence. As in previous studies, GVB were also stained by RT97, a neurofilament antibody whose epitope in tau appears to be a phosphorylated site in or near exon 2, perhaps at serine residue 46 (Brion et al. 1992). Antibodies to epitopes in the amino terminus, mid-region and carboxy terminus of tau failed to consistently stain GVB. More often they produced staining around the periphery of the GVB, giving the appearance of an “empty vacuole.” Most GVB were also immunoreactive with an antibody to ubiquitin. The results are consistent with the hypothesis that GVB are derived from sequestered altered tau possibly mediated by ubiquitin. The failure to detect most regions of tau in GVB is consistent with the idea that tau is partially degraded or highly modified in GVB.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Corticobasal degeneration ; Tau protein ; Phosphorylation ; Astrocytic plaques ; Oligodendroglial ; inclusions
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Corticobasal degeneration (CBD) is a rare, progressive neurological disorder characterized by widespread neuronal and glial accumulation of abnormal tau protein. Using immunohistochemistry we analyzed tau epitope expression and phosphorylation state in CBD and compared them to cytoskeletal changes in Alzheimer's disease (AD) and progressive supranuclear palsy (PSP). Epitopes spanning the entire length of the tau protein were present in CBD inclusions. An antibody against the alternatively spliced exon 3 did not recognize cytoskeletal lesions in CBD, but did in AD and PSP. Tau epitopes from each region of the molecule were present in cytoskeletal inclusions in CBD, including gray matter astrocytic plaques, gray and white matter threads, and oligodendroglial inclusions. As in AD, tau from CBD was highly phosphorylated. Antibodies that recognized phosphorylated tau epitopes reacted with material from CBD in a highly phosphatase-dependent manner. Again, all types of inclusions contained phosphorylated epitopes. We conclude that abnormal tau protein in CBD comprises the entire tau molecule and is highly phosphorylated, but is distinguished from AD and PSP by the paucity of epitopes contained in the alternatively spliced exon 3.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Key words Dementia ; Lewy body ; Neuropathology ; Synuclein ; Western blotting
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The identification of the α-synuclein gene on chromosome 4q as a locus for familial Lewy-body parkinsonism and of α-synuclein as a component of Lewy bodies has heralded a new era in the study of Parkinson’s disease. We have identified a large family with Lewy body parkinsonism linked to a novel locus on chromosome 4p15 that does not have a mutation in the α-synuclein gene. Here we report the clinical and neuropathological findings in an individual from this family and describe unusual high molecular weight α-synuclein-immunoreactive proteins in brain homogenates from brain regions with the most marked neuropathology. Distinctive histopathology was revealed with α-synuclein immunostaining, including pleomorphic Lewy bodies, synuclein-positive glial inclusions and widespread, severe neuritic dystrophy. We also discuss the relationship of this familial disorder to a Lewy body disease clinical spectrum, ranging from Parkinson’s disease to dementia with psychosis.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 71 (1986), S. 38-45 
    ISSN: 1432-0533
    Keywords: Alzheimer's disease ; Pick bodies ; Neurofibrillary degeneration ; Thioflavine-S ; Immunocytochemistry
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Pick body-like inclusions are described in the granular neurons of the dentate fascia in Alzheimer's disease. The inclusions are round, argyrophilic and stained by thioflavine-S. Immunocytochemically they contain antigenic determinants of neurofilaments and of Alzheimer neurofibrillary tangles. Ultrastructurally they are composed primarily of 15–18 nm straight filaments similar to the neurofibrillary pathology of progressive supranuclear palsy and Pick's disease. The dentate fascia inclusions, as well as cerebellar plaques but not amyloid angiopathy, are found most frequently in association with severe neurofibrillary degeneration.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0533
    Keywords: Monoclonal antibody ; Immunostaining ; Granulovacuolar degeneration ; Alzheimer's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A monoclonal antibody, raised against extracts from Alzheimer brain, that recognizes a phosphorylated epitope in high molecular weight neurofilament proteins and tau proteins also immunostains Alzheimer neurofibrillary tangles, neurites in senile plaques and granulovacuolar degeneration. This result suggests that granulovacuolar degeneration may contain phosphorylated proteins, possibly due to autophagy of phosphorylated perikaryal proteins that appear to be increased in Alzheimer's disease.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 76 (1988), S. 101-106 
    ISSN: 1432-0533
    Keywords: AIDS ; Confronting cylindrical cisterns ; Cytomembranous inclusions ; Tubuloreticular inclusions ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Ultrastructural studies of cells and tissues in the acquired immunodeficiency syndrome (AIDS) have revealed two distinct cytomembranous inclusions referred to as “tubuloreticular inclusions” (TRI) and “confronting cylindrical cisterns” (CCC). TRI are found most often in leukocytes and endothelial cells in conditions with elevated levels of alpha-interferon, such as viral infections, autoimmune diseases and certain neoplasms. On the other hand, CCC are detected almost exclusively in mononuclear inflammatory cells and are limited to a few conditions, of which AIDS is the most common. CCC have been proposed as an ultrastructural marker for human immunodeficiency virus (HIV) infection. We describe CCC in mononuclear inflammatory cells in the brain of a patient with AIDS. Finding CCC in brain tissue with no other specific feature such as multinucleated giant cells, nevertheless, should alert the neuropathologist to the possibility that the patient might have AIDS.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Aprosencephaly ; Glio-ependymal cyst ; Pigmented epithelial cyst ; Rathke's cleft cyst
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Aprosencephaly is a very rare brain malformation that occurs in isolated and sydromatic forms. The syndromatic form has been named “XK-aprosencephaly”, and is characterized by near total absence of prosencephalon with a midline oculofacial defect similar to the most severe forms of holoprosencephaly, in association with limb and genital anomalies. We present a case of syndromatic aprosencephaly with absence of thumb and abnormal external genitalia. A previously undescribed finding was a Tathke's cleft cyst. Two other cystic structure were also identified — an ependymal cyst, which may represent a dorsal cyst as in holoprosencephaly, and a pigmented epithelial cyst, which may represent a rudimentary eye. Additional findings were extensive calcific vasculopathy in the rudimentary prosencephalon, absence of pituitary gland, forking of the aqueduct of Sylvius and marked cerebellar hypoplasia. Since calcific vasculopathy is a common accompaniment of other inflammatory diseases of the central nervous system, its presence in this case suggests that destructive processes may be involved in the genesis of some cases of aprosencephaly.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0533
    Keywords: AIDS ; Immunohistochemistry ; gp41 ; Microglia ; Distribution
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Among 100 brains from patients with acquired immunodeficiency syndrome (AIDS), 33 brains (21 adults and 12 children) with histological evidence of subacute AIDS encephalitis were immunostained with one of the most sensitive antibodies to HIV-1 antigen, anti-gp41. Twenty-six (20/21 adults, 6/12 children) of the 33 brains showed pg41 positivity. Brains from children had fewer gp41-positive cells than brains from adults. The distribution of gp41-positive cells was characteristic. They were frequently detected and most numerous in the globus pallidus (medial 〉 lateral). Although gp41-positive cells were prevalent, fewer were detected in the corpus striatum and thalamus. Of infratentorial areas involved, the ventral midbrain, especially the substantia nigra, and the dentate nucleus contained many positive cells. Lower levels of infections, often patchy, were noted in the cerebral and cerebellar white matter and pontine base. Gp41-positive cells were rarely seen in the cerebral cortex, medulla, spinal cord, leptomeninges, choroid plexus, ependyma, subependymal areas and endothelia. Besides immunoreactive macrophages and multinucleated cells, gp41-positive microglia with various morphological alterations were abundant in the deep cerebral gray matter, ventral midbrain and dentate nucleus. Most of these microglia were undetectable with conventional histological methods. We discuss the significance of the distribution of HIV-1-infected cells, especially microglia, with respect to cellular tropism and involvement of deep gray matter nuclei in a pattern reminiscent of a multisystem atrophy.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 80 (1990), S. 493-498 
    ISSN: 1432-0533
    Keywords: Alzheimer's disease ; Amyloid ; HLA-DR ; Microglia ; Senile plaque
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Cerebellar amyloid deposits in Alzheimer's disease were studied by immunocytochemistry and with a series of antibodies that recognize human microglia, including anti-HLA-DR, LN-1, Leu-M5 and leukocyte common antigen. Microglia formed a dense reticular array throughout the cerebellum in areas with and without amyloid deposits. In areas with compact and reticular amyloid deposits, microglia had morphological features consistent with activation, such as cytoplasmic swelling and shortening and thickening of cell processes. In areas with diffuse amyloid deposits, microglia had delicate and highly ramified processes. Nevertheless, microglial cells or their processes were detected in association with amyloid deposits of all morphological types. These results raise the possibility that microglia may play a fundamental role in the pathogenesis of amyloid deposition in the cerebellum in Alzheimer's disease.
    Type of Medium: Electronic Resource
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